Clinical Characteristics of Primary Hypophysitis – A Single-Centre Series of 60 Cases

Abstract

Objective Clinical data on primary hypophysitis are still scarce. Especially non-surgical cases are underreported. We sought to analyse clinical characteristics of primary hypophysitis, particularly in clinically diagnosed patients. Design Retrospective single centre study in 60 patients with primary hypophysitis. Methods Symptoms, MRI, histopathological findings, treatment and outcomes were analysed in 12 histopathologically and 48 clinically diagnosed patients. Diagnostic criteria for clinical diagnosis were: a) MRI findings compatible with primary hypophysitis; b) course of disease excluding other differential diagnoses. Mean duration of follow-up was 69 months. Results Female sex was predominant (73%). Fatigue (52%), headache (38%) and diabetes insipidus (38%) were the most frequent symptoms. 42% had a concomitant autoimmune disease. The corticotropic, thyrotropic, gonadotropic, somatotropic axis was impaired in 67%, 57%, 52%, 20%, respectively. Men had a higher number of impaired hormone axes (p=0.022) with the gonadotropic axis being affected more frequently in men (p=0.001). Infundibular thickening (56%) and space occupying lesions (46%) were typical MRI findings. Pituitary size was frequently enlarged at presentation (37%) but diminished during observation (p=0.029). Histopathologically and clinically diagnosed cases did not differ. Conclusions The cohort of clinically diagnosed patients did not differ from our histopathologically diagnosed patients or from published cohorts with predominantly surgical patients. Thus, diagnosis of primary hypophysitis using clinical criteria seems feasible.

Hypophysitis, the Growing Spectrum of a Rare Pituitary Disease

“…Classically, hypophysitis presents with symptoms related to pituitary deficiencies with or without headaches and vision changes related to the mass effect of an enlarged pituitary gland and infundibulum. Approximately half of patients with primary hypophysitis present with headaches, while 10% to 30% present with visual disturbances related to mass effect ( Table 2 ) ( 12 , 24-40 ).…”

“…Generally, the presence of both anterior pituitary dysfunction and DI is a clue to an inflammatory/infiltrative process, craniopharyngiomas, or metastasis and is highly unlikely in pituitary adenomas. In LHy and immunotherapy-induced hypophysitis, inflammatory process predominantly affects corticotrophs, followed by gonadotrophs and thyrotrophs ( 24 ). Importantly, this pattern is distinct from that of pituitary adenomas, where corticotrophs are usually affected last ( 4 , 41 ).…”

“…Contrast uptake is usually intense, homogeneous, and less frequently heterogeneous ( 43 ). An enlarged pituitary resembles a pituitary macroadenoma in approximately half of patients with primary hypophysitis ( 24 , 30 , 44 ). While the sellar floor may be eroded in pituitary adenomas, it is usually intact in hypophysitis ( 45 ).…”

“…Clinical signs and symptoms should guide a decision to manage patients solely by observation. In patients with mild to moderate headache, mild pituitary dysfunction, no mass effect on optic chiasm, and probable LHy, observation may be safely considered ( 24 , 37 ). Initial clinical surveillance can be performed with pituitary MRI at 3 to 6 months.…”

Pituitary in the Spotlight

“…Medical therapy of patients with acromegaly within the German Acromegaly Registry [20] and an observational study on the use of somatostatin receptor agonists are topics that address acromegaly [21]. Finally, an original study on hypophysitis [22] and a review on glucocorticoid replacement therapy in primary and secondary adrenal insufficiency in Covid-19 times closes the issue [23]. This special issue of Experimental and Clinical Endocrinology and Diabetes provides the interested reader with the unique opportunity to get an exceptional overview on current diagnosis and treatment of pituitary tumors written by international experts.…”